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Abdelfatah Elasfar a,, Ahmed Khalifa b, Abdulaziz AlGhamdi c, Rizwan Khalid d,

Mohamed Ibrahim e, Tarek Kashour f


af

Prince Salman Heart Center, King Fahad Medical City, Riyadh;

af

Saudi Arabia

Metastatic cardiac tumors are far more common than primary tumors, and benign primary cardiac tumors are
common than malignant tumors. We report a 22-year-old Saudi woman with right femur osteosarcoma who was found
to have a large right ventricular mass by transthoracic and transesophageal echocardiography. Diagnosis was highly
suggestive by cardiac magnetic resonance imaging (MRI) and fluorodeoxyglucose positron emission tomography/
computed tomography (FDG PET/CT) scan. We performed a review of the literature for metastatic osteosarcoma of the
right ventricle.
2012 King Saud University. Production and hosting by Elsevier B.V. All rights reserved.

Abbreviations: RV, right ventricle, TTE, transthoracic echocardiography, TEE, and transesophageal echocardiography, MRI, magnetic resonance imaging, FDG, fluorodeoxyglucose, PET, positron emission tomography, CT,
computed tomography.
Keywords: Cardiac tumors, Intracardiac metastasis, Cardiac osteosarcoma

Introduction

he heart may rarely be affected by primary or


secondary tumors. Secondaries may occur by
local extension or haematogenous spread [8,6].
Osteosarcoma very rarely metastasizes to the
heart. About 27 cases in the last 55 years have
been reported in the literature so far. However,
the authors were unable to find any published
description of a secondary intracardiac osteogenic
sarcoma exactly like the one reported in this report, although, there have been rare lesions with

somewhat similar features in the literature [1].


Very rarely, cardiac tumors are incidentally discovered in asymptomatic patients [3]. We report
a rare case of asymptomatic metastatic osteosarcoma to the right ventricle (RV) with a review of
the literature.

Case report
A 22-year-old Saudi woman was diagnosed with
high grade right femur osteosarcoma. She had

Received 19 February 2012; revised 17 March 2012; accepted 23 May 2012.


Available online 2 June 2012

Corresponding author. Address: P.O. Box 59046, Riyadh 11525, Saudi


Arabia. Tel.: +966 50 1852566.
E-mail address: elasfar_egy@hotmail.com (A. Elasfar).

P.O. Box 2925 Riyadh 11461KSA


Tel: +966 1 2520088 ext 40151
Fax: +966 1 2520718
Email: sha@sha.org.sa
URL: www.sha.org.sa

10167315 2012 King Saud University.


Production and hosting by Elsevier B.V. All rights reserved.
Peer review under responsibility of King Saud University.
URL: www.ksu.edu.sa
http://dx.doi.org/10.1016/j.jsha.2012.05.003

Production and hosting by Elsevier

CASE REPORT

Asymptomatic metastatic osteosarcoma to the


right ventricle: Case report and review of the
literature

40

ELASFAR ET AL
ASYMPTOMATIC METASTATIC OSTEOSARCOMA TO THE RIGHT VENTRICLE: CASE REPORT AND REVIEW
OF THE LITERATURE

J Saudi Heart Assoc


2013;25:3942

CASE REPORT
Figur. 1. Transthoracic echocardiography showing RV mass. (A) Without and (B) with color superimposition.

Figure 2. Transesophageal echocardiography showing RV mass. (A) Showing one mass and (B) showing 2 lobules.

limb-sparing surgery shortly after diagnosis followed by adjuvant chemotherapy. She did not
have any cardiac complaints. During her elective
admission under oncology, she had trans-thoracic
echocardiography for pre-chemotherapy assessment and a well-defined RV mass was found
(Fig. 1A and B). Differential diagnosis included tumor or thrombus.
Trans-esophageal echocardiography confirmed
that the mass was attached to the RV free wall
with a broad base. The mass was homogenous
with multiple lobules and measured 4.5  3.2 cm
(Fig. 2A and B).
Cardiac MRI with and without contrast was
done. It showed a large mass in the RV. It was
arising from the RV free wall and was occupying
almost half of the RV (mid and apical cavity). It
had irregular edges with intermediate enhance-

ment on T1 images and is hyperintense on T2 stir


images. There was some evidence of contrast uptake on T1 weighted contrast images. It did not
seem to have a significant fatty component on T1
weighted images with fat saturation. The magnetic
resonance imaging (MRI) features were consistent
with tumorous involvement of the RV (Fig. 3A and
B).
A transthoracic echocardiography performed
4 months before the current admission was normal with no masses detected in the RV. This ruled
out the possibility of a primary tumor from the
heart and also indicates rapidly progressing mass
in the RV.
Right ventricular biopsy through a femoral approach was performed.
Five biopsies were taken; the histopathology
revealed thrombus and myocardium with no

Figure 3. Cardiac MRI showing the RV mass. (A) Without contrast and (B) with contrast.

ELASFAR ET AL
ASYMPTOMATIC METASTATIC OSTEOSARCOMA TO THE RIGHT VENTRICLE: CASE REPORT AND REVIEW
OF THE LITERATURE

41

CASE REPORT

J Saudi Heart Assoc


2013;25:3942

Figure 4. FDG/PET/CT scan showing RV mass. (A) Showing high uptake in the RV, (B) showing whole body image with high uptake in the RV,
and right knee.

evidence of tumor which was attributed most


likely to failure to pick up samples from the tumour mass. To improve diagnostic yield of the
biopsy, echocardiography guidance can be used
with 3-D transesophageal echocardiography
(TEE) or intracardiac echocardiography.
Whole body fluorodeoxyglucose positron emission tomography/computed tomography (FDG
PET/CT) scans were obtained. It revealed that
the RV mass showed intense increased FDG activity. The mass was highly suspicious for metastatic
deposits. This lesion was along with other metastasis involving inguinal, deep femoral, popliteal
and right hilar lymph nodes (Fig. 4A and B).
The patient had no cardiac symptoms, and was
being considered for second line chemotherapy.
This patient had a poor overall prognosis, therefore, the decision was not to perform cardiac surgery and closely follow the patient clinically and
with serial echocardiography. If obstruction symptoms manifested, then surgery would be strongly
considered.

Discussion
Osteosarcoma is a malignant tumor that most
frequently involves the appendicular long bones
of young adults within the second and 3rd decades of life [9]. The common demographic features for metastatic osteosarcoma includes
female sex, second and third decade of age at
the time of diagnosis and longer interval to onset
of secondary disease [9]. The clinical characteristics involve hemodynamic compromise or precordial abnormality, with magnetic resonance
imaging emerging as the gold standard for diagnosis [9]. The first report of MRI findings of cardiac osteosarcomas was presented in 2001 by
Yamagishi et al. [11] in which the tumour appeared as a huge mass of heterogeneous SI in
the LA. The report focused on the role of MRI in
differentiating malignant from benign tumors

based on a broad-based attachment and invasive


features which are similar to some extent to our
case. Since then, a few cases have been reported
with MRI images, however, with no detailed
description of MRI findings and no contrast
enhancement study included [7,5].
In our case FDG-PET/CT scan was a powerful
diagnostic tool for detecting tumour mass in the
RV along with other metastases and enhances
the level of confidence in diagnosis of tumour
spread. Also, 3-D echo has improved the detection, and characterization of cardiac tumors. Contrast echo is useful in differentiating thrombus
from tumor, and highly vascular tumors like
angiosarcomas from other less vascular tumors.
Osteosarcoma very rarely metastasizes to the
heart. Very few have been reported to occur in
the right ventricle, all of them in the RV outflow
tract. To our knowledge, no cases have been reported to occur in the RV apex and free wall and
to be discovered before being symptomatic exactly
like our case scenario. Platonov et al., has reported
one case of metastatic osteosarcoma with the tumor mass attached to the tricuspid valve [9]. The
size of metastatic osteosarcoma varies at presentation from small accidentally discovered nodules to
huge mass filling the entire right ventricle and
extending into the pulmonary valve and across
the tricuspid valve and into the right atrial cavity
[10]. Diagnosis in some of these cases was made
during investigation for severe cardiac failure
and in most of them at autopsy [10]. Very few
cases diagnosed incidentally and were asymptomatic like our case [3].
Although surgery is generally contraindicated in
the presence of metastatic disease, the role of surgical palliation of intracardiac tumor to relieve
obstruction and to prevent embolization is nevertheless valid [10]. However, even with successful
surgical resection with or without chemotherapy,
the prognosis of patients with osteosarcoma remains poor [10]. Cardiac involvement is a strong

42

ELASFAR ET AL
ASYMPTOMATIC METASTATIC OSTEOSARCOMA TO THE RIGHT VENTRICLE: CASE REPORT AND REVIEW
OF THE LITERATURE

CASE REPORT

predictor of disease elsewhere and mandates


careful surveillance, with surgical management
likely providing the best outcome. No reports for
surgical interventions for metastatic asymptomatic RV masses as in our case. Iyigun et al., reported 17-year-old female with recurrent
metastatic osteosarcoma into the left ventricle
5 months after surgical removal of the first LV
metastasis. Based on the collaborative decision,
chemotherapy was initiated and in 2 months the
size of the recurrent tumors had diminished [4].
The diagnosis of cardiac tumors with endomyocardial biopsy has been rarely described. There
are reports of failure to get enough tissues to diagnose tumor masses. The use of two-dimensional
echocardiography simultaneously with fluoroscopy has been reported as an aid to positioning
of biopsy bioptome [2].
The unique features in our case was the location of the mass in the distal to mid part of the
RV and despite being large mass, the patient is
still completely asymptomatic from cardiac point
of view. Anticoagulation has to be initiated in
such patients with proven thrombus on top of
the tumor by biopsy. There is also debate about
the best time to intervene surgically in this clinical scenario.

Disclosures
None.

J Saudi Heart Assoc


2013;25:3942

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[9] Platonov M, Turner R, Mullen J, Noga M, Welsh R.
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